CASE OF THE MONTH

Pemphigus vulgaris is rare in childhood,Dr. Jeffrey Callen said.Boy tested positive for pemphigus antibodies on direct immunofluorescence and the indirect immunofluorescence was greater than 1:640. His chest x-ray was normal.

BANFF, ALTA. — Pemphigus vulgaris is rare in childhood, Dr. Jeffrey Callen said in a poster session at the annual meeting of the Society for Pediatric Dermatology. The disease usually occurs between 40 and 60 years of age.

Dermatologically, oral lesions are often the initial manifestation. They are followed by erosions and crusts with or without flaccid blisters on the skin. The diagnosis is often made in the oral stage; treatment may result in prevention of the cutaneous blistering.

After biopsy revealed pemphigus vulgaris, this patient was sent to the division of dermatology at the University of Louisville Health Sciences Center for management.

At the initial consultation, the patient’s lesions were limited to the oral cavity Dr. Callen and his associates noted mild, non-inflammatory acne.

The boy tested positive for pemphigus antibodies on direct immunofluorescence and the indirect immunofluorescence was greater than 1:640. His chest x-ray was normal.

Biopsy revealed marked “tombstoning” of the basal keratinocytes. Acantholytic cells were observed in the suprabasilar cleft.

Initial therapy of prednisone 60 mg/day led to partial improvement, but Cushingoid features, including an acne flare, occurred early in the course. The boy had developed some skin lesions, but the primary problem remained his intraoral disease, said Dr. Callen, who is chief of the university’s division of dermatology.

The boy seemingly failed dapsone, methotrexate, and azathioprine therapies, but his compliance to drug administration was questionable. Mycophenolate mofetil also did not seem to help.

During his first semester in college, the patient attempted suicide while on low doses of prednisone. He was then given a 6-month course of monthly high-dose intravenous immune globulin in combination with daily prednisone (15-30 mg) and azathioprine (300 mg). He was able to taper the prednisone slightly during the intravenous immune globulin therapy He is now taking azathioprine 300 mg/day and prednisone 15 mg/day and is receiving intramuscular gold 50 mg/week.

“This patient illustrates some of the difficulties in dealing with serious disease in adolescents,” Dr. Callen observed. “His suicide attempt is mirrored by his lack of taking medications that might improve his condition. In addition, he has difficulties keeping appointments, which is another symptom of his abnormal psyche.”

The federal government spent about $10 billion last year on drugs covered under Medicare Part B, with one rheumatoid arthritis treatment accounting for about 5% of the spending, according the Centers for Medicare and Medicaid Services. Dr. Herb B. Kuhn, ……

Payment for Part B drugs.The federal government spent about $10 billion last year on drugs covered under Medicare Part B, with one rheumatoid arthritis treatment accounting for about 5% of the spending, according the Centers for Medicare and Medicaid Services. Dr. Herb B. Kuhn, ……

Dermatomyositis.Background Dermatomyositis occurs infrequently, with an incidence probably less than 1 per 300,000 per year. It is characterised by an intramuscular microangiopathy mediated by a complement membrane attack complex. There is a loss of muscle capillaries, muscle ischaemia, muscle-fibre necrosis ……

Peptimmune has announced that it has initiated a Phase I clinical trial to test the safety of PI-0824 in patients with pemphigus vulgaris (PV). The study is being undertaken at four sites. PI-0824 is a synthetic injectible peptide being developed ……

Kawasaki-like syndrome seen with carbamazapine. (Case Report).BARCELONA, SPAIN — Severe Kawasaki-like syndrome developed in an 8-year-old girl following the initiation of treatment with carbamazepine for partial seizures. The child was hospitalized with a 20-day history of fevers spiking to from 390 C to 400 C, abdominal ……

Aspreva Pharmaceuticals Corporation is currently working to provide more evidence-based medicines for less common diseases. The company recently announced the initiation of a Phase III trial to evaluate the safety and efficacy of CellCept (mycophenolate mofetil) with corticosteroids to achieve ……

HLA typing identifies pemphigus risk factors.ZURICH — The human leukocyte antigen class II DRB1*0402 allele proved to be the strongest genetic risk factor for pemphigus vulgaris in both whites and IndoAsians in the largest HLA typing study to date conducted in a European population, Dr….

IVIG pay, access issues confirmed.Two new reports from HHS confirm that Medicare payments for intravenous immune globulin (WIG) are severely lagging behind price increases from manufacturers, making it difficult for hospitals and physicians to offer the therapy. In an April report, the HHS Inspector ……

Placental disease causes most high MSAFP cases. (Expert Opinion).SARASOTA, FLA. — The first clinical sign of antiphospholipid syndrome may be a second-trimester elevation in maternal serum alpha-feroprotein, Dr. Anthony M. Vintzileos said at a symposium on high-risk pregnancy sponsored by Symposia Medicus. “In my view the most common ……

The authors in this study injected littermates of neonatal athymic nude mice with pemphigus vulgaris (PV) to determine whether a cholinergic agonist can abolish PV IgG-induced acantholysis. Previous studies have determined the importance of cholinergic receptors in the pathogenesis of ……

About IPPF

The International Pemphigus & Pemphigoid Foundation’s most important objectives are to provide patients and doctors worldwide with information about pemphigus and pemphigoid, and to provide patients and their caregivers much needed comfort and support so they can continue to live active, productive lives.Read more »